急性早幼粒细胞白血病诱导缓解后继发噬血细胞综合征1例并文献复习  

Hemophagocytic lymphohistiocytosis after induction remission of acute promyelocytic leukemia： one case and literature review

作　　者：陈伟[1] 孔庆玲[1] 曹江[1] 齐昆明[1] 潘秀英[1] 李振宇[1] 徐开林[1] 

机构地区：[1]徐州医学院附属医院血液科,江苏徐州221002

出　　处：《徐州医学院学报》2016年第6期379-381,共3页Acta Academiae Medicinae Xuzhou

基　　金：国家自然科学基金（81300441）;江苏省“六大人才高峰”资助项目（2013-WSN-080）

摘　　要：目的探讨急性早幼粒细胞白血病（APL）诱导缓解后合并噬血细胞综合征（HPS）的诊断及治疗要点。方法对1例诊断APL患者的临床特点进行分析，并探讨其可能机制。结果确诊病例为1例52岁男性患者，在采用亚砷酸联合维甲酸治疗4天后出现维甲酸综合征（RAS），经糖皮质激素治疗后好转，诱导缓解第24天复查骨髓提示原发病缓解，第31天白细胞及血小板恢复正常。在原发病缓解后出现持续发热，临床缺乏明确感染证据，且抗感染治疗效果不佳，后出现全血细胞减少、淋巴结及肝脾增大、血清铁蛋白明显升高、三酰甘油明显升高、NK细胞比例降低、骨髓中可见噬血现象，根据HLH-2004标准诊断符合HPS。结论绝大多数APL可临床治愈，其主要风险为诱导缓解期间严重出血，极少数患者在采用维甲酸及亚砷酸诱导期后可并发HPS，并可能与分化综合征有关。对APL原发病缓解而仍反复发热、临床缺乏明确感染证据时及时复查骨髓细胞学，排除罕见HPS可能。Objective To discuss the diagnosis and treatment of hemophagocytic lymphohistiocytosis （HLH） after remission of acute promyelocytic leukemia （APL）. Methods Retrospective analysis was performed on one APL patient, while possible mechanism was discussed. Results The patient was a 52 year - old man, who suffered from retinoic acid syndrome （RAS） after three days of treatment with arsenious acid combined with retinoic acid. His condition was relieved after glucocorticoid therapy. On Day 24 of induction remission, his bone marrow smear demonstrated APL bade been re- lieved. On Day 31, both white blood cells and platelets recovered to the normal level. Then, however, persistent fever appeared without any clinical indication of infection and the efficacy of anti - infection treatment was poor. Later, the pa- tient presented pancytopenia and hepato - splenomegaly, and produced increased levels of serum ferritin and triglycerides but a decreased proportion of NK cells, in addition to hemophagocytosis in the bone marrow. This patient was diagnosed with HLH, according to HLH -2004 diagnostic criteria. Conclusion Most APL patients can be cured. Their main risk is severe bleeding during induction remission. Very few patients suffer with HLH after induction period, which may be as- sociated with differentiation syndrome. When APL patients manifested persistent fever without clinical indication, the bone marrow should be re- examined to exclude rare HPS.

关 键 词：急性早幼粒细胞白血病 噬血细胞综合征 维甲酸综合征 

分 类 号：R733.7[医药卫生—肿瘤]